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Craniosynostosis

Gillette Children’s Specialty Healthcare is among the region’s leading centers for diagnosing and treating babies with craniosynostosis. Our Center for Craniofacial Services team treats all types of deformities affecting the skull and facial bones, giving us an unmatched depth of expertise. We use advanced surgical techniques to improve our patients’ health and physical appearance.

Why Choose Gillette?

We can determine if a baby’s abnormal skull shape is due to craniosynostosis or another less severe condition—usually without invasive testing.

Our surgeons perform more procedures to correct craniosynostosis than any other surgeons in the region do.

We offer integrated diagnostic and surgical services in a facility designed for children and families.

Our innovative team is pioneering the latest craniofacial treatments and technologies—such as transfusion-less craniosynostosis repair surgery—which reduce recovery time and improve results.

Craniosynostosis Definition

Craniosynostosis occurs when one or more of the fibrous joints in a baby’s skull (called sutures) close too early. The condition is sometimes noticeable at birth, but it also can be diagnosed as infants grow and develop. Children with craniosynostosis may have an abnormal skull shape, abnormal forehead shape, or asymmetrical eyes and/or ears.

If not corrected, craniosynostosis can create intracranial pressure (pressure inside the skull), which can lead to developmental delays or permanent brain damage. Left untreated, most forms of craniosynostosis can have profound results, including death.

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Incidence and Types of Craniosynostosis

Craniosynostosis is one of the most common physical deformities in infants, occurring in about one of every 2,000 births.

There are four types of craniosynostosis:

Scaphocephaly

The most common type of craniosynostosis, scaphocephaly is caused by the fusing of the sagittal suture, which runs from front to back down the middle of the skull. Characteristics include a long, narrow shape from front to back (narrow from ear to ear) and an overall boat-shaped appearance.

Trigonocephaly

Trigonocephaly is caused by fusing of the metopic suture, which runs from the top of the head to the middle of the forehead, toward the nose. Characteristics include a triangular-shaped forehead and eyes that are abnormally close together.

Plagiocephaly

Anterior plagiocephaly is the premature fusing of one of the coronal sutures, which extend from ear to ear over the top of the head. The forehead and brow appear pushed backwards, and the eyes are shaped differently.

Because this type of craniosynostosis causes flatness on the affected side of the head, it is often confused with deformational plagiocephaly (flat head syndrome)—a condition that doesn’t require surgical correction. Our craniofacial team works closely with families to diagnose and treat both conditions. Proper diagnosis is key to correctly managing either condition.

Brachycephaly

Brachycephaly results when both sides of the coronal sutures fuse prematurely. Characteristics include a wide-shaped head with a short skull. The fusion prevents the entire forehead from growing forward, creating a tall and flattened appearance.

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Craniosynostosis Causes and Risk Factors

In some families, craniosynostosis appears to be an inherited trait caused by a genetic mutation. So far, however, research can’t confirm that. The likelihood that a sibling of a child with craniosynostosis also will have the condition is slim—about 4 percent at most. There is also a small chance that a child born with craniosynostosis will have children with the condition.

When craniosynostosis appears as part of congenital conditions such as Crouzon or Apert syndrome, however, there is a 50-percent chance that the condition—and its associated craniosynostosis—will pass from parent to child. Our craniofacial team has extensive experience diagnosing and treating such conditions. Often, craniosynostosis is only one of several craniofacial and developmental concerns associated with an underlying condition.

Craniosynostosis Symptoms and Effects

No matter the type of craniosynostosis, the condition’s most obvious symptoms include an abnormal skull shape, abnormal forehead shape or asymmetrical eyes and/or ears.

Because many babies born vaginally have misshapen heads early in their lives, the condition is often missed. If a baby’s head shape doesn’t resolve, or remains asymmetrical, craniosynostosis is a possibility.

The condition also can create intracranial pressure (pressure inside the skull), which can lead to developmental delays or permanent brain damage if the condition isn’t surgically corrected.

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Diagnosing Craniosynostosis

Craniosynostosis is sometimes noticeable at birth, but it can also be diagnosed as infants grow and develop. Our craniofacial team evaluates babies suspected of having craniosynostosis, to confirm the diagnosis and develop a comprehensive treatment plan.

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Craniosynostosis Treatments

With the exception of very mild cases, babies born with craniosynostosis require surgery—called cranial vault remodeling—at 4 to 8 months. Usually, only one surgery is required to separate the sutures, reshape the bones and place them in the proper position. About 10 percent of children will need a second surgery, to continue the remodeling for optimal outcomes.

During surgery, a craniofacial surgeon and neurosurgeon work together to correct abnormal head shape and give the brain enough room in the skull to grow. In rare cases, a child may need a cranial remodeling orthosis, such as a Gillette CranioCap® orthosis, to complete the correction. Children also might need therapy services to improve neck mobility and strength; address feeding, swallowing or speech issues; and determine if other developmental delays exist.

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Our Craniosynostosis Services

Treatment of craniosynostosis requires care from an interdisciplinary team of experts. As one of the region’s leading facilities for correcting this condition, Gillette provides comprehensive services throughout the diagnosis and treatment process.

Specialties and services often involved in craniosynostosis care include: